Defective lysosomal arginine transport in juvenile Batten disease (Q24292865)

L-arginine transmembrane transport

GOA release 2020-03-11

1 reference

Isolation of a novel gene underlying batten disease, CLN3

GOA release 2020-03-11

author name string

Denia Ramirez-Montealegre

series ordinal

1

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David A Pearce

series ordinal

2

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language of work or name

English

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publication date

1 December 2005

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published in

Human Molecular Genetics

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volume

14

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issue

23

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page(s)

3759-73

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cites work

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Spectrum of mutations in the Batten disease gene, CLN3

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CLN3, the protein associated with batten disease: structure, function and localization

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Cross-species homology of the CLN3 gene

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BTN1, a yeast gene corresponding to the human gene responsible for Batten's disease, is not essential for viability, mitochondrial function, or degradation of mitochondrial ATP synthase.

21 January 2018

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The subcellular location of the yeast Saccharomyces cerevisiae homologue of the protein defective in the juvenile form of Batten disease

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Action of BTN1, the yeast orthologue of the gene mutated in Batten disease

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CLN3 protein regulates lysosomal pH and alters intracellular processing of Alzheimer's amyloid-beta protein precursor and cathepsin D in human cells

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Elevated lysosomal pH in neuronal ceroid lipofuscinoses (NCLs)

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A role in vacuolar arginine transport for yeast Btn1p and for human CLN3, the protein defective in Batten disease

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The transport of cationic amino acids across the plasma membrane of mammalian cells

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Purification and characterization of human lysosomes from EB-virus transformed lymphoblasts

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Brain lysosomal hydrolases in neuronal ceroid-lipofuscinoses.

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Two species of lysosomal organelles in cultured human fibroblasts

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Separate and shared lysosomal transport of branched and aromatic dipolar amino acids

21 January 2018

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Characteristics of cystine counter-transport in normal and cystinotic lysosome-rich leucocyte granular fractions

21 January 2018

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pH effects on cystine transport in lysosome-rich leucocyte granular fractions

21 January 2018

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Cystine transport in purified rat liver lysosomes

21 January 2018

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Biosynthesis and intracellular targeting of the CLN3 protein defective in Batten disease

21 January 2018

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Characterization of Cln3p, the gene product responsible for juvenile neuronal ceroid lipofuscinosis, as a lysosomal integral membrane glycoprotein

21 January 2018

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Tissue expression and subcellular localization of CLN3, the Batten disease protein

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Batten disease: evaluation of CLN3 mutations on protein localization and function

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Membrane topology of CLN3, the protein underlying Batten disease

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Infantile type of so-called neuronal ceroid-lipofuscinosis. Histological and electron microscopic studies

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Lysosomal storage of subunit c of mitochondrial ATP synthase in Batten's disease (ceroid-lipofuscinosis)

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Batten disease and the ATP synthase subunit c turnover pathway: raising antibodies to subunit c.

21 January 2018

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Abnormal degradative pathway of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid-lipofuscinosis (Batten disease).

21 January 2018

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Specific storage of subunit c of mitochondrial ATP synthase in lysosomes of neuronal ceroid lipofuscinosis (Batten's disease)

21 January 2018

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Microglial function in human APOE3 and APOE4 transgenic mice: altered arginine transport

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Arginine deficiency affects early B cell maturation and lymphoid organ development in transgenic mice.

21 January 2018

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Nitric oxide, cell signaling and cell death

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Arginine metabolism: nitric oxide and beyond

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Arginine metabolism and the synthesis of nitric oxide in the nervous system

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CAT2 arginine transporter deficiency significantly reduces iNOS-mediated NO production in astrocytes

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The role of nitric oxide in soman-induced seizures, neuropathology, and lethality.

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Lithium inhibits the modulatory effects of morphine on susceptibility to pentylenetetrazole-induced clonic seizure in mice: involvement of a nitric oxide pathway.

21 January 2018

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Nitric oxide (NO) and convulsions induced by pentylenetetrazol

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Inhibition of NO synthase increases the severity of kainic acid-induced seizures in rodents.

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NG-nitro-L-arginine, a nitric oxide synthase inhibitor, and seizure susceptibility in four seizure models in mice.

21 January 2018

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Agmatine exerts anticonvulsant effect in mice: modulation by alpha 2-adrenoceptors and nitric oxide

21 January 2018

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Altered amino acid levels in sera of a mouse model for juvenile neuronal ceroid lipofuscinoses

21 January 2018

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Sucrose-induced vacuolation results in increased expression of cholesterol biosynthesis and lysosomal genes

21 January 2018

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Interaction among Btn1p, Btn2p, and Ist2p reveals potential interplay among the vacuole, amino acid levels, and ion homeostasis in the yeast Saccharomyces cerevisiae

21 January 2018

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Diverse and essential roles of mammalian vacuolar-type proton pump ATPase: toward the physiological understanding of inside acidic compartments

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Lysosome and lysosome-related organelles responsible for specialized functions in higher organisms, with special emphasis on vacuolar-type proton ATPase

21 January 2018

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The vacuolar (H+)-ATPases--nature's most versatile proton pumps

21 January 2018

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Specific sorting of the a1 isoform of the V-H+ATPase a subunit to nerve terminals where it associates with both synaptic vesicles and the presynaptic plasma membrane

21 January 2018

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Drug-induced parkinsonism: cinnarizine and flunarizine are potent uncouplers of the vacuolar H+-ATPase in catecholamine storage vesicles

21 January 2018

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An efficient method for routine Epstein-Barr virus immortalization of human B lymphocytes

21 January 2018

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Lentivirus vector-mediated expression of tumor-associated epitopes by human antigen presenting cells.

21 January 2018

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Leucocyte intracellular pH and Na+/H+ exchanger isoform-1 activity in postpartum women with pre-eclampsia.

21 January 2018

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Mitochondrial ATP synthase subunit c storage in the ceroid-lipofuscinoses (Batten disease)

21 January 2018

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7 January 2021

Characterization of a transport system for anionic amino acids in human fibroblast lysosomes

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7 January 2021

Isolation of a novel gene underlying Batten disease, CLN3. The International Batten Disease Consortium

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7 January 2021

Brain amino acid levels are related to seizure propensity in the gerbil (Meriones unguiculatus)

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7 January 2021

Specific delay of degradation of mitochondrial ATP synthase subunit c in late infantile neuronal ceroid lipofuscinosis (Batten disease)

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDI406

7 January 2021

Arginine availability controls the N-methyl-D-aspartate-induced nitric oxide synthesis: involvement of a glial-neuronal arginine transfer.

1 reference

https://api.crossref.org/works/10.1093%2FHMG%2FDDI406

7 January 2021

Characterization of the amiloride-sensitive Na+-H+ antiport of human neutrophils.

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https://api.crossref.org/works/10.1093%2FHMG%2FDDI406

7 January 2021

The neuronal ceroid-lipofuscinoses

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7 January 2021